CAJ | 학술논문

目的：分析儿童激素耐药型肾病综合征（SRNS）伴不可逆性脑白质病变的诊断和治疗。方法回顾性分析1例确诊为SRNS伴不可逆性脑白质病变患儿的临床、实验室及影像学资料，并复习相关文献。结果患儿临床诊断SRNS后给予激素、免疫抑制剂、血液透析等治疗10个月，病程中患儿反复出现抽搐、视力下降、高血压，头颅MRI显示双侧枕顶叶以及右侧额颞叶长T2信号，T2-Flair相可见双侧枕顶叶低信号影，提示脑软化灶伴胶质增生。结论多种原因可能诱发儿童脑白质病变，在SRNS治疗过程中需高度警惕不可逆性脑白质病变的发生。
목적：분석인동격소내약형신병종합정（SRNS）반불가역성뇌백질병변적진단화치료。방법회고성분석1례학진위SRNS반불가역성뇌백질병변환인적림상、실험실급영상학자료，병복습상관문헌。결과환인림상진단SRNS후급여격소、면역억제제、혈액투석등치료10개월，병정중환인반복출현추휵、시력하강、고혈압，두로MRI현시쌍측침정협이급우측액섭협장T2신호，T2-Flair상가견쌍측침정협저신호영，제시뇌연화조반효질증생。결론다충원인가능유발인동뇌백질병변，재SRNS치료과정중수고도경척불가역성뇌백질병변적발생。
Objectives To analyze the diagnosis and management of steroid-resistant nephrotic syndrome (SRNS) accompanied with irreversible leukoencephalopathy in children. Methods The clinical, laboratory and imaging data were retrospectively analyzed in a SRNS child accompanied with irreversible leukoencephalopathy. A literature review was performed. Results After clinical diagnosis of SRNS, glucocorticoid, immunosuppressant, and hemodialysis were administrated for 10 months. During the course of treatment, the seizures, visual problems, and hypertension were repeatedly occured. The cranial MRI showed bilateral occipital parietal lobe hyperintensity and right frontotemporal lobe hyperintensity on T2-weighted imaging and bilateral occipital parietal lobe hypointensity on T2-Flair imaging, which indicated that encephalomalacia was accompanied with gliosis. Conclusions A variety of reasons may induce leukoencephalopathy in children. The accompanied irreversible leu-koencephalopathy should be strongly considered in management of SRNS.